Gorlin-Goltz syndrome (GGS) consists of ectodermal and mesodermal abnormalities. In this case report we will investigate lower extremity lesions of GGS. A 52-year-old man with GGS underwent skull and lower extremity computer tomography. Radiographic findings included cervical spondylosis, transparent areas with slurred margins, and cerebral falx calcification. Tibial and fibular specific cortic...

Patient 1 is a 34-year-old man who was referred to us for multiple basal cell carcinoma (BCC) in 2013. He was diagnosed with medullablastoma at age 3 and had his first BCC at age 12 which was surgically excised. Clinicopathological correlation supported Gorlin-Goltz syndrome as a diagnosis, but no further follow up was given. He is under dental follow up since the age of 20 for multiple odontog...

The orthokeratinized odontogenic cyst (OOC) is a rare developmental odontogenic cyst that has been considered as a variant of the keratocystic odontogenic tumour (KCOT) until Wright (1981) defined it as a different entity. Surgery is the usual treatment, and recurrence or association with Gorlin-Goltz syndrome has rarely been described. In this report, we presented three cases of this pathology...

Focal dermal hypoplasia (FDH) is a rare genetic skin disorder. The inheritance of FDH or Goltz-Gorlin syndrome is X-linked dominant and the disease is associated with a PORCN gene mutation. This gene plays a key role in the Wnt pathway, which has an impact on embryonic development. Every tissue derived from meso- and ectoderm can be affected. Patients suffer from cutaneous, ocular, osseous, ora...

Ovarian fibromas are the most common benign solid ovarian tumors, which are often difficult to diagnose preoperatively. Ovarian fibromas, especially in bilateral cases, may be cases of Gorlin-Goltz syndrome (GGS), a rare autosomal dominant disorder with predisposition to basal cell carcinomas (BCCs) and other various benign and malignant tumors. This case report describes a 25 year-old female w...

Basal cell carcinoma (BCC) is the most common non-melanoma skin cancer world wide. UV exposure plays a significant role in the pathogenesis of this tumor that is more common in older people. In the past, BCC in people younger than 30 years of age have been seen in connection with genetic diseases like Gorlin-Goltz syndrome. The histological type was mostly superficial and most common localizati...

Introduction: Dentigerous cysts (DCs) are the most common developmental cysts of the jaws, mostly associated with impacted third molars and canines. Multiple or bilateral DCs are rare and typically occur in association with some syndromes including cleidocranial dysplasia and Gorlin-Goltz. The occurrence of multiple DCs is rare in the absence of these syndromes. Case Presentation: A 28-year-ol...

Dear Editor, Nevoid basal cell carcinoma syndrome (NBCCS; also called Gorlin-Goltz syndrome, MIM: 109400) is a rare autosomal dominantly inherited disorder caused by defects in the hedgehog signaling resulting in constitutive pathway activity and tumor cell proliferation.1 In addition to basal cell carcinoma from a young age, distinguishing features are keratocystic odontogenic tumors, dyskerat...